From Volume 46, Issue 5, May 2019 | Pages 450-461
Systemic sclerosis, although a rare disease, has numerous direct and indirect effects on the oral cavity. This article aims to inform clinicians of the signs and symptoms they may see in patients with the disease and the impact it can have on dental management. The main effect, microstomia, is not just limited to systemic sclerosis, thus the clinical and laboratory techniques described may be helpful when managing other patients with limited mouth opening.
Systemic sclerosis (SSc) is a rare autoimmune disease of unknown aetiology, characterized by excessive deposition of collagen in the skin and multiple internal organs, fibrosis of blood vessels and numerous immunologic abnormalities.1 Its prevalence is estimated at 20 cases per 1 million population, with a predominance in females.2 Also known as scleroderma, the disease can present with a wide clinical picture that varies greatly in severity and prognosis. Clinical forms range from limited cutaneous systemic sclerosis, where there is skin involvement only, to forms where there is severe and often progressive involvement of multiple internal organs, known as diffuse cutaneous systemic sclerosis, and occasionally a fulminant course (fulminant systemic sclerosis).3
CREST syndrome (Calcinosis, Raynaud's disease, oEsophageal dysmotility, Sclerodactyly and Telangiectasia) is an older term used to describe limited cutaneous systemic sclerosis with involvement of the face and neck. Diffuse cutaneous systemic sclerosis refers to skin thickening proximal to the knees and elbows as well as distally, as in limited cutaneous systemic sclerosis, and involvement of the face. Internal organs that are most commonly involved are the gastrointestinal tract, respiratory, renal, cardiovascular, musculoskeletal, endocrine and genito-urinary systems3 (Table 1).
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